An Incomplete Pentalogy of Cantrell

Pentalogy of Cantrell is a rare entity of congenital defects involving the abdominal wall,sternum, diaphragm, pericardium and heart. The complete pentalogy and its variants havebeen described in the literature. We report a 47-day-old girl with an incomplete pentalogy ofCantrell presenting with congenital absence of the sternum, congenital heart defects, and anepigastric hernia. Two even rarer expressions of this disorder, tricuspid atresia type IIc andasymmetric kidneys, were also found. The patient was successfully managed with palliativepulmonary artery banding as preparation for a modified Fontan operation when she is older.We report this rare case, and discuss the pathologic findings and surgical strategy.